Characteristics of patients with Wilson disease in the United States: An insurance claims database study

BACKGROUND Wilson disease(WD)is a progressive,potentially fatal degenerative disease affecting the liver and central nervous system.Given its low prevalence,collecting data on large cohorts of patients with WD is challenging.Comprehensive insur-ance claims databases provide powerful tools to collect retrospective data on large numbers of patients with rare diseases.AIM To describe patients with WD in the United States,their treatment and clinical outcome,using a large insurance claims database.METHODS This retrospective,longitudinal study was performed in the Clarivate Real-World Data Product database.All patients with≥2 claims associated with an Interna-tional Classification of Diseases 10(ICD-10)diagnostic code for WD(E83.01)between 2016 and 2021 were included and followed until death or study end.Patients were divided into two groups by whether or not they were documented to have received a specific treatment for WD.Clinical manifestations,hospital-isations,liver transplantation and death were documented.RESULTS Overall,5376 patients with an ICD-10 diagnostic code for WD were identified.The mean age at inclusion was 41.2 years and 52.0%were men.A specific WD treatment was documented for 885 patients(15.1%),although the number of patients taking zinc salts may be underestimated due to over the counter purchase.At inclusion,the mean age of patients with a documented treatment was 36.6±17.8 years vs 42.2±19.6 years in those without a documented treatment.During follow-up,273 patients(5.1%)died.Compared with the American general population,the standardised mortality ratio was 2.19.The proportion of patients with a documented WD-specific treatment who died during follow-up was 4.0%and the mean age at death 52.7 years.CONCLUSION Patients treated for WD in the United States had an excess early mortality compared with the American population.These findings indicate that there is a significant unmet need for effective treatment for WD in the United States.

Zinc and Silver Salts-Containing Lamellar Titanium Phosphate: A Multifunctional Filler

Zinc and silver compounds have been studied because they have ultraviolet light barrier properties and bactericidal action, respectively. Materials with multifunctional characteristics have been sought to produce polymeric nanocomposites. In this work, the chemical modification of titanium phosphate (TiP) was carried out through a route with successive intercalations. TiP was synthesized and consecutively pre-expanded with ethylamine and pyromellitic acid. Then it was modified with zinc acetate and silver nitrate. The final product was characterized by Fourier transform infrared spectroscopy, differential scanning calorimetry, wide-angle X-ray diffractometry, field emission scanning electron microscopy coupled with energy dispersive X-ray spectroscopy and thermogravimetry. Infrared revealed dislocation and appearance of bands according to the intercalating agent. Inorganic salts interfered in the crystallization and melting processes of pyromellitic acid. Vanishing of the TiP hkl plane and variation and appearance of new crystallographic planes at low diffraction angles induced intercalation. SEM showed agglomerated structures. New thermal degradation events at higher temperatures endorsed the formation of zinc and silver carboxylate salts. We concluded that a new miscellaneous and multifunctional matter was achieved.

Ag-doped ZnO nanorods synthesized by two-step method

A two-step method is adopted to synthesize Ag-doped ZnO nanorods. A ZnO seed layer is first prepared on a glass substrate by thermal decomposition of zinc acetate. Ag-doped ZnO nanorods are then assembled on the ZnO seed layer using the hydrothermal method. The influences of the molar percentage of Ag ions to Zn ions （RAg/zn） on the structural and optical properties of the ZnO nanorods obtained are carefully studied using X-ray diffractometry, scanning electron microscopy and spectrophotometry. Results indicate that Ag ions enter into the crystal lattice through the substitution of Zn ions. The （002） c-axis-preferred orientation of the ZnO nanorods decreases as RAg/Zn increases. At RAg/Zn 〉 1.0%, ZnO nanorods lose their c-axis-preferred orientation and generate Ag precipitates from the ZnO crystal lattice. The average transmissivity in the visible region first increases and then decreases as RAg/Zn increases. The absorption edge is first blue shifted and then red shifted. The influence of Ag doping on the average head face, and axial dimensions of the ZnO nanorods may be optimized to improve the average transmissivity at RAg/Zn 〈 1.0%.

Investigation of the By-products Formed during the Catalytic Synthesis of 4,4＇-Methylenedimethyldiphenylcarbamate

The structures of the by-products formed during the catalytic synthesis of 4,4＇-methyldiphenylcarbamate （MDC） by the reaction of 4,4＇-methylenedianiline （MDA） with dimethyl carbonate （DMC） were identified and then the mechanisms of their formation were proposed.

Investigation of the Crystallization Kinetics of Zn(Met)(AcO)_2·H_2O in Mixed Solution of Water and Acetone by Microcalorimetry

The crystal growth process of Zn(Met)(AcO)2旽2O from the mixed solution of water and acetone has been investigated using a Calvet microcalorimeter. The heat produced and the rate of heat production during the crystal growth process at 298.15, 301.15, 304.15, 307.15 and 310.15 K have been measured. On the basis of experimental and calculated results, the thermodynamics parameters (the apparent activation enthalpy, the activation entropy, and the activation free energy), the rate constant and the kinetic parameters (the activation energy, the pre-exponential factor) during the crystal growth process have been obtained. The results show that the crystal growth proceeds in accordance with the Burton-Cabrera-Frank dislocation theory.